Neuro Clinical

Front 1

Intial pathology in Alzheimer's is where in the brain?

Back 1

hippocampus and entorhinal cortex

Front 2

Features of Lewy Body Dz

Back 2

mild parkinsons, early dementia
hallucinations
REM sleep d/o

Front 3

what are lewy bodies made of?

Back 3

alpha synuclein (intranuclear inclusions)

Front 4

What are the 3 types of frontotemporal lobar degeneration?

Back 4

Frontal Temporal Dementia
Primary Progressive Aphasia
Semantic Dementia

Front 5

Characteristics/location of frontal temporal dementia

Back 5

Mostly frontal lobes
personality changes, impaired social conduct, disinhibited and perseverative behavior

Front 6

Characteristics/location of primary progressive aphasia?

Back 6

left frontal lobe
non-fluent speech but preserved understanding (Broca's aphasia)

Front 7

Characteristics/location of semantic dementia?

Back 7

temporal lobes
loss of understanding...fluent, meaningless speech (wernicke's aphasia)

Front 8

What are Pick bodies?

Back 8

inclusion bodies which stain for tau protein found in frontal temporal lobe degeneration dzs.

Front 9

Tx for ALS?

Back 9

Riluzole and supportive

Front 10

UMN vs LMN signs

Back 10

UMN - hyper reflexia
LMN - fasiculations and atrophy

Front 11

How does ALS present?

Back 11

UMN and LMN signs
normal sensory and intellect
usually asymmetric

Front 12

Genetics of Spinal muscular atrophy?

Back 12

AR (Survival motor neuron on 5q)

Front 13

Werdnig Hoffman Syndrome signs/sxs?

Back 13

SMA-1
LMN sxs
floppy baby
tongue fasiculations

Front 14

Kennedy Dz genetics and sxs? lifespan?

Back 14

spinobulbar muscular atrophy
X-linked CAG repeat androgen receptor defect
LMN w/gyecomastia
lifespan is normal

Front 15

Dx of Guillain-Barre syndrome? presentation?

Back 15

increased protein in CSF
sxs: ascending weakness, arreflexia

Front 16

What disease is:
distal greater than proximal weakness, foot drop, atrophy and numbness

Back 16

Charcot-Marie-Tooth dz
most common is uniform demyelination

Front 17

What is multiple mononeuropathy suggestive of?

Back 17

nerve infarction/inflammation
vasculitic neuropathies (lupus/polyarteritis nodosa)

Front 18

How do you diagnose a small fiber neuropathy?

Back 18

biopsy (not EMG or NCS)

Front 19

Tx for Guillain Barre?

Back 19

no roids
give IVIg and plasma exchange

Front 20

Dx for Chronic Inflammatory Demyelinating Polyradiculoneuropathy?

Back 20

EMG shows conduction block and temporal dispersion

Front 21

Name the disease:
acute onset of arm pain associated with patchy weakness and numbness
cannot form "OK"

Back 21

Parsonage-Turner Syndrome

Front 22

What other conditions is Parsonage-Turner associated with?

Back 22

DM, SLE, vasculitis

Front 23

Which neuromuscular junction dz is presynaptic? postsynaptic?

Back 23

pre-synpatic - Lambert Eaton, Botulism
post-synaptic - Myasthenia Gravis

Front 24

What tumor is associated with myasthenia gravis?

Back 24

thymoma

Front 25

inheritance pattern of congenital myasthenic syndromes?

Back 25

AR

Front 26

Histologic difference btw LEMS and MG

Back 26

LEMS - increased post-synaptic folds
MG - decreased post-synaptic folds

Front 27

Which disorders have proximal weakness more than distal weakness?

Back 27

NM junction disorders
and
myopathies

Front 28

What are typical findings in myopathies?

Back 28

elevated CK or CPK
and
proximal weakness

Front 29

usefull tests and results for myopathies?

Back 29

EMG - short duration/small amplitude APs
CK/aldolase - elevated
Bx - metabolic deposits

Front 30

Pathophys of muscular dystrophies?

Back 30

reduced or absent expression of protein product of dsytrophin (X-linked)

Front 31

Features of Duchenne musc dyst?

Back 31

calf pseudohypertrophy
gowers sign
cardiomyopathy
mental retardation

Front 32

How is Becker's musc dyst different from Duchenne?

Back 32

less MR
less cardiomyopathy
later onset
normal lifespan

Front 33

Histopath of IBM? Tx?

Back 33

beta-amyloid deposits (alzheimer's of the muscle)
no tx

Front 34

Diagnostic tests for toxic myopathies?

Back 34

check CK
(often a normal EMG)

Front 35

3 examples of mitochondiral myopathies

Back 35

MERRF - myoclonic epilepsy, ragged red fibers
MELAS - mitoch encephalomyopathy lactic acidosis stroke
MNGIE - mitoch neurogastrointestinal encephalopathy syndrome

Front 36

Family history of DM, short stature, sz, deafness indicates what disease?

Back 36

mitochondrial myopathies

Front 37

What are ragged red fibers?

Back 37

accumulations of abnormal mitochondria

Front 38

How do mitochondrial myopathy pts present?

Back 38

ptosis, ophthalmoplegia

Front 39

When do you get periodic paralyses?

Back 39

after large carbohydrate meals and rigorous exercise, stress

Front 40

HYPOkalemic paralysis
vs
HYPERkalemic paralysis

Back 40

HYPO - Ca channel, hrs to days tx with acetazolamide
HYPER - Na channel, mins to hrs

Front 41

Which brain tumors are ass w/ NF1?

Back 41

pilocytic astrocytomas of the optic pathways and cerebellum

Front 42

which brain tumors are ass w/ NF2?

Back 42

bilateral acoustic schwannomas

Front 43

Where do brain mets come from?

Back 43

lung > breast > melanoma > colon

Front 44

Treatment of Choice for multiple brain mets?

Back 44

whole brain radiation

Front 45

Treatment for single brain met?

Back 45

resection (gamma if not resectable) followed by whole brain rad

Front 46

Imaging modality for brain tumors?

Back 46

MRI w/ and w/o contrast

Front 47

Treatment for low grade gliomas?

Back 47

Maximum Surgical resection

Front 48

Treatment for high grade glioma?

Back 48

Resection + RT + (temozolomide)

Front 49

What predicts better survival in pts with high grade gliomas?

Back 49

younger age, methylated MGMT gene

Front 50

Treatment for medulloblastoma?

Back 50

resection + craniospinal RT + chemo

Front 51

What type of tumor is a medulloblastoma and what are the symptoms?

Back 51

primitive neurectodermal tumor. HA, N/V, ataxia

Front 52

What are rosenthal fibers associated with?

Back 52

pilocytic astrocytomas (eosinophilic corkscrew fibers), alexander dz?

Hint 52

None

Front 53

How is a cluster HA different from a migraine?

Back 53

clusters are shorter, regular patterns, faster onset, piercing pain

Front 54

Episodic vs. Chronic tension or migraine HA?

Back 54

chronic is on more than 15 days/month

Front 55

You must have what sxs during a HA to classify as a migraine?

Back 55

either N/V or photophobiaANDphonophobia

Front 56

When is the migraine prodrome? Aura?

Back 56

prodrome - hours before HA…aura - minutes before

Front 57

What is the most important risk factor for cluster HA?

Back 57

Smoking

Front 58

What is the occurrence pattern in cluster HA?

Back 58

once or twice a day for 6-8wks. Once or twice/year

Front 59

What is the defect in FHM1?

Back 59

Ca channel disorder that leads to elevated Glu in the cortex

Front 60

What causes the pain in migraine?

Back 60

sterile trigeminal nerve inflammation

Front 61

Where is the generator/modulator in migraines?

Back 61

rostral pons and midbrain tegmentum

Front 62

What is allodynia in migraine due to?

Back 62

central sensitization

Front 63

Red flags for HA?

Back 63

Systemic,Secondary; Neurologic sxs; suddOnset; Older; Previous hx